Clinical Vignettes Part I

Abstract

Patients with sickle cell disease and/or (rarely) trait are at increased risk for developing recurrent episodes of priapism, also known as stuttering priapism, and major ischemic priapism. Treatment of acute ischemic priapism is reactive; whereas ideal management consists of preventative approaches to ultimately promote the best improvement in patient's quality of life. Leg ulcers in patients with sickle cell disease (SCD) are quite common, with ∼20 % of patients with HBSS reporting either having an active or a past ucler. They can be confused with venous ulcers, with lower extremity hyperpigmentation confounding further the diagnosis. Several factors believed to contribute to the development of leg ulcers in patients with SCD are discussed in this article. Sickle cell liver disease (SCLD) occurs because of a wide variety of insults to the liver that happen during the lifetime of these patients. SCLD includes a range of complications of the hepatobiliary system and is increasing in prevalence with the aging adult sickle population. Liver nodular regenerative hyperplasia (NRH) is more common than realized and underappreciated as a diagnosis and requires liver biopsy with reticulin staining. Undiagnosed, the insidious damage from liver NRH can lead to noncirrhotic portal hypertension or cirrhosis.

Fig. 1.

Histologic findings with 3 different stains from same area, all at 200x magnification. (A) H&E: there is subtle variation in hepatocyte size (arrows), associated with mild sinusoidal dilation. (B) Masson: no fibrosis is seen in the area of sinusoidal dilation. (C) Reticulin: the liver cell plates are narrowed (arrows) between regions where the liver cells are normal in size and the plates are 1 to 2 cells thick.

Fig. 2.

Pattern of liver enzymes elevation in the patient.